Hepatosplenic T-cell lymphoma

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive form of peripheral T-cell lymphoma that primarily affects the liver, spleen, and bone marrow. This disease is characterized by the proliferation of cytotoxic T-cells and is most commonly seen in young adult males. The etiology of HSTCL remains largely unknown, but it has been associated with chronic immunosuppression, particularly in individuals who have undergone solid organ transplantation or those with autoimmune diseases treated with immunosuppressive drugs.

Symptoms and Diagnosis

The clinical presentation of HSTCL can be nonspecific but typically includes hepatomegaly (enlarged liver), splenomegaly (enlarged spleen), and systemic symptoms such as fever, night sweats, and weight loss. Laboratory findings often show cytopenias (reduction of blood cells), elevated liver enzymes, and abnormal lymphocytes in the peripheral blood and bone marrow.

Diagnosis of HSTCL is challenging and requires a combination of clinical, laboratory, and histopathological findings. Imaging studies such as ultrasound, CT scan, or MRI can reveal the extent of organ involvement. Definitive diagnosis is made through a biopsy of the affected organ, typically the liver or spleen, which shows infiltration by atypical T-cells that are usually positive for the T-cell marker CD3 and negative for CD4 and CD8 surface markers.

Treatment and Prognosis

The treatment of HSTCL is difficult, and there is no standardized treatment protocol due to the rarity of the disease. Options may include chemotherapy, stem cell transplantation, and targeted therapies, but the prognosis remains poor with a median survival of less than 2 years. Early diagnosis and aggressive treatment are crucial to improving outcomes.

Epidemiology

HSTCL is a very rare disease, accounting for less than 1% of all non-Hodgkin lymphomas. It has a male predominance and is most commonly diagnosed in young adults, with a median age of onset between 20 and 40 years.

Pathophysiology

The pathogenesis of HSTCL is not well understood, but it is thought to involve genetic mutations that lead to the uncontrolled proliferation of cytotoxic T-cells. These cells then infiltrate the liver, spleen, and bone marrow, causing the characteristic symptoms and findings of the disease.

See Also

• Lymphoma
• T-cell lymphoma
• Non-Hodgkin lymphoma
• Immunosuppression